Haemorrhage Secondary to Cerebral Aneurysm in a Senegalese Patient with Systemic Lupus Erythematosus:A Case Report

Introduction: Hemorrhage by ruptured cerebral aneurysm is a rare and severe complication of systemic lupus erythematosus (SLE). We reported a case of this complication. Observation: A 24-year-old woman black Senegalese patient was followed in our department since for a systemic lupus with cutaneous and articular involvement and class III and V Lupus nephritis. She was readmitted for acute headache and early postprandial vomiting. The examination showed a meningeal syndrome, a subacute lupus eruption in the trunk, panniculitis and fever. The cerebral computer tomography showed spontaneous haemorrhage from saccular aneurysm. She was managed by immediate aneurysm clipping and medical treatment including bolus of methylprednisolone and immunosuppressive therapy was maintained. The outcome was favourable, but there was neurological damage such as brachial weakness. Conclusion: Hemorrhage by ruptured cerebral aneurysm is a rare and severe complication of SLE. The risk of damage is also significant. Immediate neurosurgical management and aggressive medical treatment may improve the prognosis.

Intrathecal injection with methotrexate plus dexamethasone in the treatment of central nervous system involvement in systemic lupus erythematosus

Abstract:Objective To investigate the effect of intrathecal injection (IT) with methotrexate (MTX) plus dexamethasone (DXM) in treating central nervous system involvement in systemic lupus erythematosus (CNS lupus). Methods Twenty-four CNS lupus patients that were refractory to conventional steroid therapy were selected for IT with MTX 10-20?mg plus DXM 10-20?mg. The effects and side effects of IT were closely observed. Results The symptoms and signs of 22/24 (91.7%) CNS lupus patients receiving IT improved considerably. Cerebrospinal fluid pressure,protein and WBC levels declined from 201.5±155.4?mm?H2O, 145.2±87.6?mg/dl and 25.1±14.3/mm3 to 128.7±108.1?mm?H2O, 60.8±38.3?mg/dl and 6.8±2.1/mm3 respectively. Transient side effects were observed in 4 patients: 1 with itching sensation of lower limbs, 2 with headache and 1 with incontinence.Conclusion IT with MTX plus DXM is a promising method for treating CNS lupus and deserves further investigation.

Intracranial Hypertension Syndrome in Systemic Lupus Erythematosus: Clinical Analysis and Review of the Literature

In order to better understand the clinical manifestation of systemic lupus erythematosus （SLE） with intracranial hypertension syndrome （IHS）, we analyzed the clinical features and treatment of a typical SLE patient with IHS. SLE is one of the most unpredictable autoimmune diseases involving multiple organ systems that is defined clinically and associated with antibodies directed against cell nuclei. IHS is an uncommon manifestation of neuropsychiatric SLE （NPSLE） and is characterized by an elevated intracranial pressure, papilledema, and headache with occasional abducens nerve paresis, absence of a space-occupying lesion or ventricular enlargement, and normal cerebrospinal fluid chemical and hematological constituents. IHS has been reported in a few sporadic cases in patients with SLE worldwide, but rarely has been reported in China. In this study, a 34-year-old female SLE patient with IHS was. reported and pertinent literature reviewed. The clinical presentation, image logical features, and investigatory findings were discussed.

Neurological Involvement in Systemic Lupus Erythematosus (SLE): Our Recent Experience

Introduction: The central, psychiatric and peripheral neurological manifestations of lupus are among the most severe visceral disorders and are grouped under the general term of “neuro-psychiatric systemic lupus erythematosus” (NPSLE). We conducted a cross-sectional observational study within our Department of Internal Medicine aimed at describing the clinical and evolutionary aspects of central neurological disorders of SLE, excluding lupus myelopathy. Patients and Methods: This was a retrospective and observational cross-sectional study carried out from 1 January 2015 to 31 October 2017, in the Department of Internal Medicine of Aristide le Dantec University Hospital in Dakar (Senegal). All patients hospitalized during this period who met the 1997 ACR classification criteria of SLE and who presented with a central neuropsychiatric syndrome attributable to SLE (as defined by ACR 1999) were included. Patients with isolated headache, acute myelitis or secondary neurological involvement attributable to a toxic, metabolic, infectious or tumour-related cause were excluded from our study. Results: During the study period, 10 patients with neuropsychiatric lupus involvement were treated at our institution, including 9 women and 1 man;the median age was 29 years (20 - 55 years). Neurological involvement occurred during the course of lupus evolution in 9/10 cases. The median time to SLE evolution was 18 months (0 - 60 months). Neuropsychiatric syndromes as defined by the 1999 ACR were commonly associated and more than half of our patients had multiple neuropsychiatric syndromes. There were 5 cases of confusion syndrome and coma, 4 cases of seizure, 3 cases of psychosis, 2 cases of acute cerebrovascular disease and 1 case of aseptic meningitis. Among the extra-neurological manifestations of SLE, haematological and dermatological involvements were common. Renal involvement affected half of the patients. The other manifestations were: polyarthritis in 3 patients, serositis in 2 patients, 5 cases of fever, 4 cases of deterioration of the general state, and one isolated case of ophthalmological involvement. Therapeutically, 8 patients received a bolus of methylprednisolone and 3 patients received a bolus of cyclophosphamide. Oral corticosteroids and hydroxychloroquine were administered to all patients, and azathioprine was administered in 2 patients. The evolution was favorable in 4 patients, other 2 patients maintained neurological sequelae and 2 patients were transferred to intensive care. Death was recorded in 4 patients. Conclusion: Neuropsychiatric manifestations of lupus are rare and sometimes severe, potentially life-threatening. In our patients, we have identified some of the most severe neurological syndromes according to the ACR nomenclature. The neurological involvement is exceptionally revealing, as these syndromes are often associated and integrated into a systemic context of lupus. The evolution is rapidly unfavorable and requires early diagnosis and optimal management.

Neuropsychiatric Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by multisystemic involvement and diverse manifestations. Neuropsychiatric systemic lupus erythematosus (NPSLE) is a complex neurological disorder characterized by neuropsychological dysfunction. NPSLE is associated with increased morbidity and mortality. In 1999, the American College of Rheumatology developed 19 discrete neuropsychiatric syndromes that comprised NPSLE. Nervous system disease in systemic lupus erythematosus is manifested by a wide variety of clinical manifestations. The pathogenesis of NPSLE is due to autoantibodies, neuronal and non neuronal antigens and the generation of proinflammatory cytokines and mediators. Anatomopathological lesions are attributed to in situ thrombosis, edema, hemorrhage, vasculitis, atherosclerosis or atheroembolism. The diagnosis of NPSLE remains largely one of exclusion and is approached by clinical evaluation, and supported when necessary by autoantibody profiles, diagnostic imaging, electrophysiologic studies and objective assessment of cognitive performance. Brain MRI abnormalities in NPSLE might show small punctate focal lesions in white matter being the most common MRI finding, followed by cortical atrophy, ventricular dilation, cerebral edema, diffuse white matter abnormalities, focal atrophy, cerebral infarction, acute leukoencephalopathy and intracranial hemorrhage. The treatment is based on the use of symptomatic therapies, immunosuppressives and non-pharmacologic interventions. This review paper was designed to understand the pathophysiology for better management of NPSLE.

金实教授中医药治疗系统性红斑狼疮伴中枢神经损害的临床经验

金实教授认为,系统性红斑狼疮伴中枢神经损害癫痫发作病理因素以风、痰、瘀、毒为主,常用经验方定痫汤加减;以发作性头痛为特点的中枢神经损害患者,配合经验方活血定痛汤加减;中枢神经损害症状不严重者,标本同治,以益肾化毒的狼疮方合定痫汤治疗。

新生儿红斑狼疮伴中枢神经系统损害

报告1例新生儿红斑狼疮伴中枢神经损害。患儿女,2个月。面部、躯干与四肢可见暗红色环状红斑,表面覆有少许鳞屑,部分轻度萎缩。脑部CT检查:对称性弥漫性脑部白质区密度减低。住院期间使用糖皮质激素乳膏与避光剂外用治疗。